A juxtaglomerular cell tumor (JCT) is a rare, renin-secreting tumor of the kidney and can cause hypertension. JCT is pathologically benign, and resection of the tumor is curative for hypertension. We report the case of a 17-year-old girl who had hypertension and hypokalemia for 1 year. Laboratory studies showed increased basal plasma renin activity, but normal serum aldosterone level. Abdominal computed tomography disclosed a 2 cm solid mass in the left kidney. However, renal vein sampling and captopril test results were equivocal. Partial nephrectomy was performed and histologic examination demonstrated typical features of JCT. Hypertension and hypokalemia completely resolved postoperatively. JCT should be considered when investigating hypertensive individuals with high plasma renin activity.
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